Amy Kiernan, PhD

Research Overview

Sensory organs are unique structures in the body plan of all multicellular organisms that allow perception of the environment. Our lab uses the power of mouse genetics to understand the fundamental molecular pathways involved in sensory development and disease. The eye and the inner ear of the mouse are very similar to humans and thus can serve as good models for understanding key developmental processes. As in humans, many of the specialized cell types in the ear and the eye of the mouse, such as hair cells, photoreceptors and ganglion cells, cannot regenerate when damaged due to genetics, environmental factors, or normal aging. Loss of these important cells leads to irreversible deafness, vestibular dysfunction and vision loss. Therefore, identifying the key molecules involved in the specification of these cell types and their precursors will be important when developing therapies using stem cells, progenitor cells, or gene therapy as well as in understanding the congenital defects associated with mutations in these genes. The long-term goal of this laboratory is to use advanced mouse genetic approaches, including conditional gene targeting and inducible expression systems, to elucidate key molecular pathways involved in different aspects of sensory development. Currently, our lab is studying the role of the Notch signaling pathway and the transcription factor SOX2 in the development of the eye and the inner ear. By understanding some of the basic molecular processes by which sensory organs form and are maintained, we can begin to develop therapies for prevention, repair and regeneration in sense organ disease.

Current Projects

Dissecting the molecular pathways leading to anterior segment dysgenesis (ASD) of the eye and its contribution to developmental glaucoma.

The role of the Notch ligand JAGGED1 during sensory precursor development in the inner ear

The role of the transcription factor SOX2 in the development of the prosensory lineage in the inner ear